Neuropediatrics 2017; 48(S 01): S1-S45
DOI: 10.1055/s-0037-1602961
P – Poster
Georg Thieme Verlag KG Stuttgart · New York

Severe Course of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) with Detection of an Unknown Antineuronal Antibody

S. Christ
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
F. Leypoldt
2   Universiätsklinikum Kiel, Kiel, Germany
,
B. Hanebeck
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
B. Haber
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
I. Brösse
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
C. Schmitt
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
G. Hoffmann
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
S. Syrbe
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
,
A. Ziegler
1   Universitätsklinikum Heidelberg, Heidelberg, Germany
› Author Affiliations
Further Information

Publication History

Publication Date:
26 April 2017 (online)

 

Background: CIDP is a rare autoimmune disease, regularly seen in adulthood. In addition to macrophages and B- and T-cell–mediated mechanism, novel antibodies including anti-contacin and anti-neurofascin have been identified recently, although they are detectable only in 5 to 10% of the cases. It is assumed that further, so far unknown antibodies are involved in the pathogenicity. We report on a 12-year-old girl with a severe course of CIDP.

Methods and Results: After initial treatment of a Guillain-Barré syndrome (GBS) at the age of 8 years, there was clinical deterioration at the age of 11 years with persisting pathologic findings in neurography and cytoalbumin dissociation in cerebrospinal fluid supporting a diagnosis of CIDP. Under monthly administration of intravenous immunoglobulins (IVIG), motor symptoms stabilized over the next 12 months. At the age of 12 years, there was an acute deterioration with generalized weakness, pain, dysphagia with loss of deglutition reflex, and hyporeflexia. As there was no improvement under steroid and IVIG infusion, plasmapheresis was performed and switched to immunoadsorption after the immunohistochemical identification of a so far not characterized antineuronal antibody. Additional immunosuppressive treatment with mycophenolat and rituximab was started. Under this treatment, clinical symptoms stabilized.

Conclusion: We report an atypical, severe course of CIDP in a 12-year-old girl. Our multimodal immunosuppressive therapy led finally to clinical stabilization and enabled rehabilitation. Immunohistological analyses indicate a humoral mediated pathomechanism from a novel, antineuronal antibody which requires further characterization.